Neoplasms of locomotive system Jongkolnee Settakorn, MD...

Neoplasms of

locomotive system

Jongkolnee Settakorn, MD

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Scope

• General consideration• Classification• Prognostic factors• Soft tissue tumors• Bone tumors• Joint tumors

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General consideration

Rare tumors of more than 100 subtypes

• Incidence of soft tissue tumors:– Benign: 3000/million– Malignant: 30/million

• Incidence of bone tumors:– Malignant: 8-20/million

• Common sites of soft tissue sarcoma:– Extremities (75%)– Trunk wall (10%)– Retroperitoneum (10%)

• Pattern of tumors: age, gender, site– Lipoma

• Painless, rare in hand-lower leg-foot• Uncommon in children

– Vascular tumors• >50% occurs in patients younger than 20

years– Benign soft tissue tumors

• 99% are superficial• 95% are < 5 cm in diameter

Classification

• Cells of origin, e.g.,– Bone forming cells– Cartilage forming cell– Muscle cell

• Behavior– Benign– Intermediate– Malignant

Classification of soft tissue tumors

1. Fibrous tumors2. Fibrohistiocytic tumors3. Lipomatous tumors4. Smooth muscle tumors and related

lesions5. Extragastrointestinal stromal tumors6. Skeletal muscle tumors7. Tumors of blood and lymph vessels8. Perivascular tumors

9. Synovial tumors10.Mesothelial tumors11.Peripheral nerve sheath tumors and

related lesions12.Primitive neuroectodermal tumors and

related lesions13.Paraganglionic tumors14.Extraskeletal osseous and

cartilagenous tumors15.Miscellaneous tumors

Classification of bone tumors

1. Cartilagenous tumors

2. Osteogenic tumors

3. Fibrogenic tumors

4. Fibrous histiocytic tumors

5. Ewing sarcoma and primitive neuroectodermal tumors

6. Hematopoietic tumors

7. Giant cell tumors

8. Notochordal tumors

9. Vascular tumors

10.Smooth muscle tumors

11.Lipogenic tumors

12.Neural tumors

13.Miscellaneous tumors

14.Miscellaneous lesions

15.Joint lesion

Benign tumor

• Well circumscribed border

• Cured by complete local excision

• Do not recur locally– If recur, recur in a non-destructive fashion

• Do not metastasize

• Examples:– Lipoma– Benign fibrous histiocytoma– Enchondroma

Intermediate tumor

– Associated with an infiltrative or locally destructive growth pattern

– Recur locally

• Locally aggressive tumor– Do not metastasize– Example: Desmoid fibromatosis

• Rarely metastasizing tumor – Have ability to metastasize but <2% risk– Example: Angiomatoid fibrous histiocytoma

Malignant tumor

• Local destructive growth

• Recurrence

• Significant risk of distant metastasis

• Examples:– Osteosarcoma– Ewing sarcoma

Nomenclature

• Benign: -oma or benign• Malignant: -sarcoma or malignant

• Fibroblast: fibro-• Fibroblast and histiocyte: Fibrous histiocyto-• Fat: Lipo-• Muscle: -myo-• Smooth muscle: Leiomyo-• Skeletal muscle: Rhabdomyo-

• Blood vessel, endothelial cell: hemangio- or angio

• Lymph vessel: lymphangio- , or angio• Synoviocyte: tenosynovial• Nerve fiber: neuri-, neuro-, schwanno-• Bone forming cell, osteoblast: osteo- or

ossificans• Cartilage forming cell: chondro-• Myxoid: myxo- or myxoid-

Poor prognostic factors

• Age Young• Tumor size Large• Tumor site (location) Vital organs• Depth of involvement Deep• Lymph node metastasis Positive• Distant metastasis Positive• Neurovascular involvement Positive• Clinical staging Advanced• Histologic grade High

High histologic grade

• Tumor differentiation Poorly differentiation

• Mitotic count (mitosis) >20/10 HPF

• Tumor cell necrosis >50%

• Cellularity High

• Nuclear pleomorphism Positive

• Tumor emboli Positive

Normal fatPoorly differentiated liposarcoma

Well differentiated liposarcoma

Mitoses

Tumor necrosis

Low cellularity High cellularity

Nuclear pleomorphism

Tumor emboli

Tumor staging

Soft tissue tumor

1. Fat1.1. Lipoma (benign)

– Mature adipocytes– 20-40 years old– A painless mass– Subcutaneous or deep soft tissue, 5% multiple– Gross and microscopic features:

• Well circumscribed mass of fat• Lobules of mature adipocytes

– Treatment: Excision

• Prognosis– Subcutaneous lipoma: cure– Deep (intramuscular lipoma): risk of local

recurrence

1.2. Liposarcoma (malignant)– Common sarcoma of adulthood (40-70 years)– A large mass, pain– Deep soft tissue of proximal extremities and

retroperitoneum– Subtypes

• Well differentiated LS dedifferentiated LS• Myxoid LS round cell LS• Pleomorphic LS

– Gross and microscopic features:• Well circumscribed or infiltrative, fat cut surface

(WDLS), gelatinous surface (MXLS), solid grey white surface with hemorrhage and necrosis (PMLS)

• Lipoblast– Treatment: Excision, radiation, chemotherapy– Prognosis:

• Depend on site, tumor extension, complete excision?, histologic grade, multifocal?

• Local recurrence, metastasis

Lipoblasts

WDLS

MXLS

RCLS PMLS

2. Fibrous tumors and tumor-like lesions

2.1. Reactive pseudosarcomatous proliferation

2.1.1. Nodular fasciitis (benign)

2.1.2. Myositis ossificans (benign)

2.1.3. Palmar, plantar and penile fibromatosis (benign)

2.1.4. Desmoid (aggressive fibromatosis) (intermediate)

2.2. Fibroma (benign)

2.3. Fibrosarcoma (malignant)

2.4. Fibrous histiocytic tumor

2.4.1. Benign fibrous histocytoma

2.4.2. Malignant fibrous histiocytoma

Fibrosarcoma MFH

3. Tumors of skeletal muscle

3.1. Rhabdomyoma (benign)

3.2. Rhabdomyosarcoma (malignant)– Most common malignant soft tissue tumor in

children and teenage– Rhabdomyoblast– Subtypes: embryomal, alveolar, pleomorphic

4. Tumors of smooth muscle

4.1. Leiomyoma (benign)– Uterus

4.2. Leiomyosarcoma (malignant)

5. Tumors of peripheral nerve

5.1. Schwannoma (neurilemmoma) (benign)– Large nerve– Antoni A (cellular area, verocay body)– Antoni B (acellular area)

5.2. Neurofibroma (benign or intermediate)– Small and large nerve

5.3. Malignant peripheral nerve sheath tumor

6. Tumors of vessel

6.1. Hemangioma (benign)6.1.1. Capillary hemangioma6.1.2. Cavernous hemangioma

6.2. Glomus tumor (benign)

6.3. Lymphangioma (benign)

6.4. Angiosarcoma (malignant)

6.5. Kaposi’s sarcoma (malignant)

Lymphangioma Angiosarcoma

7. Synovial sarcoma

– Mass around joint– 20-50 years– Histology: biphasic pattern of stroma and

epithelial components

1. Cystic lesions in bone

1.1. Solitary bone cyst– Simple bone cyst– Long bone

1.2. Aneurysmal bone cyst– Long bone– Blood sponge

Bone tumor

2. Bone forming tumors

2.1. Osteoma (benign)– Cortex of skull and facial bone– Mass of normal bone

2.2. Osteoid osteoma (<2 cm) (benign)

2.3. Osteoblastoma (>2 cm) (benign)– Long bone– Pain (osteoid osteoma)– Mass of osteoblasts, osteoid, trabeculae of

woven bone

Osteoid osteoma

2.4. Osteosarcoma (malignant)

– A primary intramedullary high grade malignant tumor in which the neoplastic cells produce osteoid.

– The most common, non-hematopoietic, primary malignant tumor of bone

– Incidence: 4-5 / million– Disease of the young: second decade of life– Male: female = 3:2– Site: metaphysis of long bone, especially around

the knee

– Clinical features: severe deep pain, mass, fracture

– Radiologic features:• X-ray: lytic or blastic or mixed lesion with

cortical destruction, soft tissue extension and periosteal reaction (Codman’s triangle)

• CT and MRI– Etiology: unknown, genetic susceptibility

• P53 loss of function mutation• MDM2 over expression• Germ-line mutation in RB1 gene (hereditary

retinoblastoma)

Periosteal reaction: Codman triangle

– Gross and microscopic features:• Intramedullay mass at metaphysis of long

bone• Soft tissue extension• Bone or cartilage formation• Areas of hemorrhage and necrosis• Pleomorphic malignant cells produce osteoid.

– Treatment: surgery + chemotherapy– Prognosis: 60-70% disease free survival

• A 5 year old girl with a painful mass at left leg for 4 month

• Knee disarticulation and chemotherapy

3. Cartilage forming tumors

3.1. Osteochondroma (benign)– A cartilage capped bony projection arising on

the external surface of bone, containing marrow cavity that is continuous with that of the underlying bone

– The most common bone tumor (35% of benign, 8% of all)

– Age <30 years– Multiple OC: 15%– Etiology: growth plate arrest, EXT1 gene– Site: metaphysis-diaphysis of long bone

(distal femur, proximal humerus, proximal tibia and fibula)

– Clinical features: most asymptomatic, mass

– Radiologic features: X-ray = pedunculated or sessile mass (a projection of the cortex in continuity with the underlying bone)

– Gross: mushroom shape bone mass with cartilagenous cap

– Histology: 3 layers (perichondrium, cartilagenous cap, and bone)

– Treatment: excision– Prognosis: Cure, rare recurrence, rare

malignant change (chondrosarcoma) in case with thick cap

• A 17 years old man with mass on right thigh• Excision

3.2. Chondroma, enchondroma (benign)– Diaphysis of long bone (finger)– Mass of cartilage

3.3. Chondroblastoma (intermediate)– Mass of chondroblast

3.4. Chondromyxoid fibroma (benign)– Mass of chondroid, myxoid, and fibroblast

Chondroblastoma Chodromyxoid fibroma

3.5. Chondrosarcoma (malignant)– Axial bone– 30-60 years– Malignant cells produce cartilage

4. Fibrous and fibro-osseous tumors

4.1. Fibrous cortical defect and non-ossifying fibroma (benign)– Cortex– Mass of fibroblast

4.2. Fibrous dysplasia (benign)– Medullar of long bone– Fibroblasts and trabeculae of woven bone

4.3. Fibrosarcoma and malignant fibrous histiocytoma (malignant)

Non-ossifying fibroma

Fibrous dysplasia

5. Miscellaneous tumors

5.1. Ewing sarcoma (malignant)– Malignant small round cell tumor– Diaphysis of long bone– <15 years

5.2. Giant cell tumor (intermediate)– Epiphysis of long bone (around knee)– X-ray: soap-bubble appearance– Huge multinucleated giant cells

6. Metastatic tumor

– The most common malignant tumor affecting the skeleton

– 2/3 of the patients age between 40-60 years– Primary sites: breast, lung, prostate, kidney,

thyroid, bowel, stomach, cervix– Site ~ location of the primary tumor and blood

flow• Vertebrae (lumbar spine), proximal femur, rib,

sternum, pelvis, skull, shoulder girdle• Axial bone (45%), appendicular bone (30%),

multiple bone (25%)

– Clinical features: pain, swelling, fracture, neurologic symptom

– Radiologic features:• X-ray: lytic lesion (thyroid, kidney), blastic

lesion (prostate), or mixed (breast, lung)• Bone scan: very sensitive• CT, MRI

– Histology: as primary tumor• Adenocarcinoma > squamous cell carcinoma

– Prognosis and treatment: poor, palliative

• A 70 years old man with left shoulder pain• An underlying history of colonic cancer

(adenocarcinoma)• Treatment: curettage, cementation, and internal

fixation with T-plate

Metastatic adenocarcinoma

Joint• Solid joint

• Cavitated joint– Articular cartilage– Synovial membrane (synoviocytes)– Synovial fluid– Fibrous capsule, ligament, muscle

1. Ganglion or ganglion cyst

• A small cyst (1-1.5 cm) near a joint capsule or tendon sheath, especially near the wrist joint (dorsal aspect)

• Most common mass in hand• Adult (20-40 years)• Subside in most cases• Etiology: cystic or myxoid degeneration of

connective tissue• Symptom: freely moveable mass, local pain or

weakness• Sign: more prominent with wrist flexed

• Gross and microscopic features:– Firm, fluctuant, translucent nodule– Stalk attaches cyst to tendon sheath or joint– No communication with joint space– Cyst contains mucinous material– The cyst wall lacks a true cell lining

Ganglion cyst

• Management– Aspiration

• Compression dressing for 48-72 hours• Splinting wrist for several days

– Surgical excision if symptom persist despite aspiration

• Course– Spontaneous resolution (53%)– Recurrence within 2-5 years after treatment

• After surgical excision (42%)• After aspiration (47%)

2. Synovial cyst

• Herniation of synovium through a joint capsule or massive enlargement of a bursa

• Baker cyst = synovial cyst in popliteal space

• Clinical features: mass, pain, interfering of movement

• Ultrasonography, CT, MRI• Gross and microscopic features:

– Synovial lining cyst (synoviocytes)– Synovial fluid may contain inflammatory cells

and fibrin• Treatment:

– Medication (NSAID), physical therapy– Excision

• A 40 years old woman with a mass at popliteal fossa

• Excision

3. Tumors of synoviocyte (intermediate)

3.1. Localized: Giant cell tumor of tendon sheath

3.2. Diffuse: Villonodular tenosynovitis